Glycogen storage disease (GSD) is a rela-tively rare inherited metabolic disease. However, its relative rarity implies that no metabolic centre has experience of large numbers of patients and experience with long-term manage-ment is limited. In addition, there is wide variation in meth-ods of medical treatment. With the development of medical care, life-expectancy in glycogen storage disease (GSD) has improved considerably. With ageing liver adenoma may de-velop which will bring about several complications-compres-sion, hemorrhage, or even transformation into carcinomas-and needs immediate intervention. In this paper, reviews for the mechanism and surgical intervention of GSD are presented.

BACKGROUND:RADA16 is a mature amphiphilic self-assembling peptide, which can be assembled into nanofibers, and promote MC3T3 E1 cellattachment, spreading and proliferation.
OBJECTIVE:To observe the effects of the new-type self-assembling peptide hydrogel NBD/RADA16 on osteogenic differentiation of mouse preosteoblasts MC3T3 E1.
METHODS:The MC3T3 E1 cells were inoculated in NBD/RADA16 self-assembling peptide hydrogel and RADA16 hydrogel for osteogenic induction. cells undergoing simple osteogenic induction served as controls. After culture for 1, 3, 6 days, the activity of alkaline phosphatase was detected. After 7 days, western blot assay was used to determine the expression of bone morphogenetic protein-2. After 21 days, alizarin red staining was used to observe calcified nodules.
RESULTS AND CONCLUSION:MC3T3 E1 cells grew wel on the NBD/RADA16 peptide hydrogel, which were superior to those on the RADA16 hydrogel. The activity of alkaline phoshpatase was higher in the NBD/RADA16 group than the RADA16 and control groups (P<0.01). Compared with the RADA16 hydrogel, mineralized matrix deposition and expression of bone morphogenetic protein-2 were higher on the NBD/RADA16 peptide hydrogel (P<0.01). These findings indicate that the NBD/RADA16 peptide hydrogel is superior to the RADA16 hydrogel for promoting the osteogenic differentiation of MC3T3 E1 cells.

[Objective] To explore how to deal with the paternity test of complex adoption cases. [Method] Samples from 13 families, in which adoptive parents were suspected related to biological parents, were genotyped using "Identifder + Sinofder + Powerplex 16" combined system (D8S1179, D21S11, D7S820, CSFIPO, D3S1358, TH01, D13S317, D16S539, D2S1338, D19S433, VWA, TPOX, D18S51, D5S818, FGA, D6S1043, D12S391, PentaD, PentaE) followed by further statistical analysis. [Result] Among all 13 cases, 2 were completely accordance with the Mendel law, PI > 10 000. There found more than 3 inconsistent loci in 8 cases. And found 1～2 inconsistent loci in 3 cases, needed to test more STR loci until PI≥10 000. The half sibling index (HSI) was also calculated with ITO method. The adoptive parents of 2 cases were not excluded from a full sibling with biological parents. In addition, Y-STR loci were tested for 4 cases (father/son). Two adoptive fathers of them were not excluded from the paternal relationship with biological fathers. [Conclusion] The most (76.9%) of all (13) complex adoptive cases of paternity test could be drawn a definite conclusion with combined system of "Identifder + Sinefiler + Powerplexl6". Minority (23.1%) of them was not definite yet and needed testing more STIR loci. Meanwhile, we suggested adding Y-STR tests and providing HSI for reference.

Objective To investigate the molecular mechanisms of synergistic effects of BFA and CDDP on human lung cancer GLC-82 cells, and to test the levels of PERK-ATF4 pathway. Methods GLC-82 cells were incubated with 50 ng/mL of BFA or/and 2 μg/mL of CDDP for 24 or 48 hours. The levels of PERK, p-PERK and ATF4 in GLC-82 were analyzed by real-time PCRand/or Western Blot. Results The levels of PERK were lowest in CDDP group, but higher in BFA group (P < 0.05), the highest in group of BFA+CDDP (P < 0.05 or P < 0.01). The p-PERK level decreased in group of BFA+CDDP (P < 0.05 or P < 0.01). There was no significant change of ATF4 expression in CDDP group, but ATF4 expression increased slightly in BFA group, and increased further in group of BFA+CDDP (P < 0.05 or P < 0.01)which was also higher than that in BFA group or CDDP group (P < 0.05 or P < 0.01). Conclusions The upregulated levels of PERK and ATF4 by the combination of BFA and CDDP may be one of the mechanisms of synergistic anti-cancer effect of BFA and CDDP on GLC-82 cells.

Objective To investigate the prediction value of ultrasound endometrial pattern at the day of hCG administration on clinical outcome in patients undergoing in vitro fertilization-embryo transfer (IVF-ET).Methods 679 cycles of IVF-ET conducted between January 2011 and December 2012 in Beijing Obstetrics and Gynecology Hospital were reviewed retrospectively.All cycles were divided into three groups based on the endometrial pattern measured on the hCG day:group A (n =411) with triple line endometrium,group B (n =228) with no obvious triple line endometrium,group C (n =40) with homogeneous hyperechoic endometrium.Clinical outcomes such as implantation rate,pregnancy rate and miscarriage rate were analyzed.Results Among three groups,the implantation rates were 27.05％,20.33％,19.57％,respectively (x2 =9.04,P =0.01); the pregnancy rates were 34.31％,25.00％,12.50％,respectively (x2 =12.20,P =0.00).The implantation rate and pregnancy rate in group A were higher than the other two groups (P ＜0.05).The miscarriage rates were 16.31％,19.30％,40.00％,respectively(P ＞ 0.05),there were no significant difference among them.Conclusions The ultrasound endometrium pattern on the day of hCG administration has predictive value on clinical outcome in patients undergoing in vitro fertilization-embryo transfer.

Objective To explore whether PPARαtransgenic mice are more sensitive animal models in the evalua-tion of toxicity of PPARαagonists.Methods Twenty-eight 8-week old PPARαtransgenic mice (Tg) and 28 C57BL/6J mice (WT) with half males and half females were randomly divided into high dose group (400 mg/kg of clofibrate), low dose group (30 mg/kg of clofibrate) and solvent control group (10%sodium carboxymethyl cellulose ).The time of gavage administration lasted 28 days.The blood biochemistry , organ coefficient and pathological changes of the heart , liver, kid-neyweretestedafterthedrugadministration.Thegrowthofmicewasalsorecorded.Results ①Bloodbiochemistry:Com-pared with the WT male administration group , in the Tg male administration group , the levels of blood creatinine ( CREA) and aspartate aminotransferase (AST) were markedly increased (P<0.01, P<0.05).② Organ coefficient: Compared with the Tg control group, the kidney coefficients of Tg administration group were significantly increased (P<0.01,P<0.05).③Histopathology:Compared with the WT administration group , the pathological damages of liver and kidney were more serious in the Tg administration group .Conclusions Compared with C57BL/6J mouse, PPARαtransgenic mice are more sensitive in evaluation of hepatotoxicity and nephrotoxicity of PPARαagonists .It is a new animal model .

Spontaneous intracerebral hemorrhage (ICH) has the highest rates of mortality and morbidity in stroke types.Experimental and clinical studies demonstrate that inflammatory reactions are early triggered by hematoma components,enhance the damage within the hemorrhagic brain,and influence the prognosis.Recent studied show that neutrophil to lymphocyte ratio (NLR),as an easily available biomarker to assess the systemic inflammatory status,has predictive roles in patients with ICH.This article reviews the predictive roles of NLR in patients with acute ICH.

Objective: To Evaluation the effect of PD-1 inhibitor Nivolumab on the proliferation and cytotoxicity of anti-CD19 chimeric antigen receptor T cells (CD19-CAR-T) in vitro. Methods: Five patients with high PD-1 expression in peripheral blood and five healthy volunteers were selected. These peripheral blood mononuclear cells were used as the source of T cells to prepare CD19-CAR-T cells. Different doses (72, 36, 18 μg/ml) of Nivolumab was added on day 8 to the culture medium. Patient T cells incubated with 72 μg/ml Nivolumab and CD19-CAR-T cells of healthy volunteers were used as controls. CCK-8, lactate dehydrogenase (LDH) cytotoxicity assay and ELASA were used to detect the proliferation capacity, the specific cytotoxicity and the inflammatory factor secretion. Results: ①T cells from patients with high expression of PD-1 as the source of CD19-CAR-T cells did not affect transfection rate compared with that of healthy volunteers [(32.80±7.22)% vs (35.10±5.84)%, t=-0.554, P=0.593]. ②Incubation of CD19-CAR-T cells with 72 μg/ml Nivolumab did not affect CD19-CAR-T cell proliferation, but its cytotoxicity was significantly higher than that of CD19-CAR-T cells alone or patients’ T cells +72 μg/ml Nivolumab (all P<0.001), there was no significant difference in the killing activity between the 72 μg/ml and 36 μg/ml Nivolumab treated CD19-CAR-T cells on Pfeiffer cells (P=0.281, 0.267, respectively), and they were all higher than those of 18 μg/ml Nivolumab treated CD19-CAR-T cells (all P<0.001). ③Different doses of PD-1 inhibitor Nivolumab combined with CD19-CAR-T cells does not affect the secretion of IFN-γ and IFN-α (all P>0.05). Conclusion Combination of 36 μg/ml PD-1 inhibitor and CD19-CAR-T cells could reduce the drug toxicity and enhance the cytotoxicity.

Objective: To investigate the efficacy and safety of CD19 chimeric antigen receptor T (CAR-T) lymphocytes for the treatment of B cell lymphoma. Methods: A total of 22 patients with B-cell lymphoma from February 1, 2017 to July 1, 2018 were reviewed to evaluate the efficacy and adverse reactions of CD19 CAR-T. Results: Of 22 patients with B-cell lymphoma received CD19 CAR-T cells, the median dose of CAR-T cells was 7.2 (2.0-12.0) ×10⁶/kg. Nine of 12 cases of relapse refractory patients were overall response. Complete remission (CR) occurred in 2 of 12 patients, partial remission (PR) in 7 of 12 patients. The overall response in minor residual disease positive (MRD) group was 8 of 10 patients. CD19 CAR-T cells proliferated in vivo and were detectable in the blood of patients. The peak timepoints of CAR-T cells proliferated in the relapsed refractory and MRD positive groups were 12 (5-19) and 4.5 (1-12) days after treatment respectively, and among peripheral blood cells, CAR-T cells accounted for 10.10% (3.55%-24.74%) and 4.02% (2.23%-28.60%) of T lymphocytes respectively. The MRD positive patients achieved sustained remissions during a median follow-up of 8 months (rang 3-18 months) . None of all the patients relapsed during a median follow-up time of 10 months (3-18 months) . However, 7 PR responders of the relapsed refractory patients maintained a good condition for 1.5-6.0 months. One patient bridged to hematopoietic stem cell transplantation, another one sustained remission for 12 months. Cytokine-release syndrome (CRS) occurred in 14 patients with grade 1-2 CRS in MRD positive group and grade 3 CRS in relapsed refractory group. Conclusions CAR-T cell therapy not only played a role in the rescue treatment of relapsed and refractory patients, but also produced a surprising effect in the consolidation and maintenance of B-cell lymphoma. CD19 CAR-T cells might be more effective in the treatment of MRD positive B-cell lymphoma patients than in the refractory or relapsed cases. High response rate was observed with fewer adverse reactions.

Objective: To study the characteristics of attentional bias to emotional stimulus in medical workers of emergency department and explore the relationship between the emotional Stroop effect and mood states, thus providing a reference to the assessment and intervention of emergency medical workers’ mental health. Methods: Two kinds of emotional Stroop tasks with words and pictures and the Profile of Mood States (POMS) were administered to thirty-six medical workers of emergency department and thirty-six medical workers of other departments. Results: The reaction time to negative and positive words and pictures were all higher than the reaction time to neutral stimulus, thus showing the significant Stroop effect in medical workers. The Stroop effect to negative emotional stimulus in medical workers of emergency department was significantly smaller than that in medical workers of other departments, while the difference of Stroop effect to positive stimulus was not significant between the two groups. Correlation analyses revealed that the correlations between Stroop effect size to positive pictures and tension and angry of POMS were all negatively significant (r value was -0.314 and -0.330 respectively) , and the correlation between Stroop effect size to negative pictures and fatigue was negatively significant (r=-0.303) . Conclusions There is a significant emotional Stroop effect in medical workers. Although the ability of anti-interference to emotional stimulus in medical workers of emergency department was better than medical workers of the other departments, the long-term avoidance to negative emotional stimulus was still has a negative effect on the mood states of individuals.